A 54 year old female, with history of uncontrolled Diabetes Mellitus presented with complaints of progressively severe frontal headaches with associated nausea and dizziness. CT scan of the head revealed a 10 cm frontal bone lytic lesion extending into the nasal bones with evidence of sequestrum. Mucosal thickening and opacification of the frontal sphenoid and ethmoid sinuses was also noted. MRI was consistent with CT finding and revealed further cortical destruction of frontal calvarium outer table along with para-meningeal and dural enhancement. CSF studies were negative. Patient was started on intravenous antifungal therapy with Amphotericin B lipid complex. Frontal sinus trephination with irrigation/aspiration and simultaneous diagnostic nasal endoscopy revealed no frank pus or necrosis. Aspirate’s bacterial and fungal culture were negative. Patient underwent an open incision trephination of frontal sinus that revealed destruction/moth-eaten appearance of the anterior table of the frontal sinus, biopsies were taken, No pus was encountered.

1. ATYPICAL PRESENTATION AND DIAGNOSIS OF
RHINO-CEREBRAL MUCORMYCOSIS
Fakhri Kalolwala MD, Yasir Al-Zubaidi MD, Layth Al-Jashaami MD, Christelle Kassis MD,
Maricopa Integrated Health System, Phoenix, Arizona
Introduction
Mucormycosis is a rare fungal infection, caused by
fungi belonging to the taxonomic order of Mucorales.
Mucorales species most commonly associated with
rhinocerbral mucormycosis is Rizopus.
Mucormycosis mostly seen in immunocompromised
hosts at a rate of about 500 cases in the United
States annually. major clinical forms include
rhinocerebral, pulmonary, and cutaneous.
Rhinocerebral mucormycosis develops upon
inhalation into the paranasal sinuses. The invading
fungus may spread inferiorly into the palate,
posteriorly into the sphenoid sinus, laterally into the
cavernous sinus and cranially into the brain. Initial
symptoms are consistent with sinusitis, peri-orbital
cellulitis /facial pain, numbness and progress into
vision loss, diplopia and cranial nerve palsies. A
black necrotic facial eschar is the hallmark of
mucomycosis. Bone destruction is seen, late and
only after soft tissue necrosis has occurred. Imaging
and demonstration of fungal hyphae on biopsy are
required for confirmation of diagnosis. We present a
case of atypical presentation of rhinocerebral
mucormycosis in an immunocompetent patient.
Case Report
Frontal bone pathology was relevant for chronic
osteomyelitis, devitalized bone with nonseptate
branching hyphae morphologically suggestive of
Mucor. Antifungal therapy was continued. Patient
underwent anterior craniofacial resection consisting
of bifrontal craniectomy, as well as resection of
orbital bar, and cribriform plate, debridement of skull
base, cranialization of frontal sinus and harvest of
pericranial pedicled flap for vascular reconstruction
of skullbase. Post-op, patient improved and was
discharged on oral Posaconazole. Repeat MRI two
month later reveal no residual of recurrent disease.
Case Report Continued
References
A 54 year old female, with history of uncontrolled
Diabetes Mellitus presented with complaints of
progressively severe frontal headaches with
associated nausea and dizziness.
CT scan of the head revealed a 10 cm frontal bone
lytic lesion extending into the nasal bones with
evidence of sequestrum. Mucosal thickening and
opacification of the frontal sphenoid and ethmoid
sinuses was also noted. MRI was consistent with CT
finding and revealed further cortical destruction of
frontal calvarium outer table along with para-
meningeal and dural enhancement. CSF studies
were negative. Patient was started on intravenous
antifungal therapy with Amphotericin B lipid complex.
Frontal sinus trephination with irrigation/aspiration
and simultaneous diagnostic nasal endoscopy
revealed no frank pus or necrosis.
Aspirate’s bacterial and fungal culture were negative.
Patient underwent an open incision trephination of
frontal sinus that revealed destruction/moth-eaten
appearance of the anterior table of the frontal sinus,
biopsies were taken, No pus was encountered.
Discussion
A high index of suspicion, in this patient with
uncontrolled diabetes, prompted the initiation of
adequate antifungals and the performance of a
frontal sinus biopsy despite negative diagnostic
nasal endoscopy and frontal sinus aspiration. Direct
microscopic examination/stains and fungal cultures
of tissue specimen remains the standard methods for
mucormycosis diagnosis confirmation. Mucorales are
typically broad, ribbonlike, nonseptate with branching
arising at right angle. Newer molecular diagnostic
techniques notably PCR are promising.
Successful treatment consist of aggressive surgical
resection/debridement in combination with
appropriate antifungals and reversal of underlying
predisposing factors.
(1) Walsh TJ, Gameletsou MN, McGinnic MR,
Hayden RT, Kontoyiannis DP. Early clinical and
laboratory diagnosis of invasive pulmonary,
extrapulmonary, and disseminated Mucormycosis
(zygomycosis). Clin Infect Dis. 2012 Feb; Suppl
1:S55-60. Doi: 10.1093/cid/cir868
(2)Spellberg B, Edwards J Jr, Ibrahim A. Novel
Perspective on Mucormycosis: pathophysiology,
presentation and management. Clin Microbiol Rev
2005: 18:556-69
(3)Petrikkos G, Skiada A, Lortholary O, Roilides E,
Walsh TJ, Kontoyiannis DP. Epidemiology and
Clinical Manifestations of Mucormycosis, Clin Infect
Dis 2012; 54 (Suppl 1): S23-34
(4)Glazer M, Nusair S, Breuer R, Lafair J, Sherman
Y, Berkman N. The Role of BAL in the diagnosis of
pulmonary mucormycosis. Chest: 117:279-82
(5)Hayden RT, Quin X, Procop GW, Roberts GD,
Lloyd RV. In situ hybridization for the identification of
filamentous fungi in tissue section. Diagn Mol Pathol
2002; 11:119-26.
Even though usually seen in immunocompromised
patient s with hematologic malignancies,
Mucomycosis is described in immonocompetent
patients. Predisposing conditions include
uncontrolled diabetes, iron overload, major trauma
and prolonged steroids use. Mucormycosis is
associated with high mortality rate, thus timely
recognition, imaging and tissue diagnosis is key to
early initiation of treatment and improved outcome.
Our patient atypical clinical presentation consisted of
worsening headache with no vision, skin or sinus
symptoms. For early detection of sinus lesions,
Computerized Tomography (CT) has been a key
advance over conventional radiographs. In fact, a
timely CT head revealed evidence of frontal bone
destruction and diffuse sinus disease.
Pathology
Discussion ContinuedImaging
Abnormal signal and enhancement along the frontal calvarium with
cortical destruction of the outer table
Lytic lesion of the frontal bone
Pansinus mucosal thickening
Frontal sinus bone biopsy-Mucor, GMS Stain.
Frontal bone Osteomyelitis. Mucor.
Frontal Dura, excision Biopsy, Mucor.